Mononeuritis Multiplex as Initial Manifestation of Pure Neuritic Leprosy-A Forgotten Cause: Clinical, Electrodiagnostic and Pathologic Correlations.

Despite being common, polyneuropathy remains a diagnostic challenge for most clinicians. Mononeuritis multiplex (MM) refers to involvement of several or many peripheral nerves at the same or different points in time by a disease process. This report describes a case of an atypical presentation of Hansen's disease (HD) as mononeuritis multiplex in the left lower limb with corresponding radiographic, electrodiagnostic, and histopathological data that confirmed pure neuritic leprosy (PNL). We reiterate that although the incidence of PNL is exceedingly low characterized by nerve involvement without the characteristic cutaneous stigmata, leprosy is still the commonest cause of MM in the Indian sub-continent.This report underscores the crucial need for a heightened multi-disciplinary awareness of this "forgotten and uncommon" presentation of PNL. It is imperative that the treating physician should also understand the various neurological presentations, both mimics and chameleons, of this treatable disease to prevent permanent neuropathic injury and disability.

Polineuropatia desmielinizante como manifestação inicial isolada de hanseníase: relato de caso / Demyelinating polyneuropathy as an isolated initial manifestation of leprosy: case report

RESUMO: Modelo do Estudo: Relato de caso. Importância do problema: No mundo, mais de três milhões de pessoas estão vivendo com deficiência física devido à hanseníase. O Brasil é o segundo país com o maior número de casos novos registrados.A magnitude e o alto risco de incapacidade mantêm a doença como problema de saúde pública. O diagnóstico de hanseníase em geral é simples. Porém, quadros com ausência de lesões cutâneas características, somente com alterações neurais, representam um desafio para o diagnóstico diferencial com outras doenças neurológicas. Comentários: Relatamos o caso de um paciente encaminhado ao serviço de neurologia com história clínica e eletroneuromiografia compatíveis com polineuropatia desmielinizante, sem qualquer lesão cutânea ao exame de admissão. O raciocínio clínico inicial foi direcionado para o diagnóstico das polineuropatias desmielinizantes inflamatórias adquiridas como Polineuropatia Desmielinizante Inflamatória Crônica (CIDP) e suas variantes. No entanto, após anamnese e exame físico detalhados, chamou a atenção a ausência do componente atáxico e a presença predominante de alterações sensitivas de fibra fina, espessamento de nervo e importante fator epidemiológico para hanseníase, motivando a suspeita e a in-vestigação desta enfermidade por meio da biópsia de nervo que foi sugestiva de hanseníase. Após três meses, em novo exame do paciente para biopsiar áreas de anestesia para reforçar o diagnóstico, observou-se o surgimento de extensas lesões levemente hipocrômicas no tronco e membros inferiores, cuja biópsia definiu o diagnóstico de hanseníase. (AU)

ABSTRACT: Study: Case report. Importance: Worldwide over three million people are living with disabilities due to leprosy. Brazil is the second country with the highest number of new cases registered. The magnitude and high risk of disability make the disease a public health problem. The diagnosis of leprosy can be simple. However, in the absence of skin lesions and with many possibilities of neurological impairment, diagnosis can become a challenge. Comments: We report the case of a patient referred to the neurology service with a clinical history and electrophysiological tests compatible with demyelinating polyneuropathy, without any skin lesion at admission examination. The initial clinical research was directed to the diagnosis of acquired inflammatory demyelinating polyneuropathies such as Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)and its variants. However, after anamnesis and detailed physical examination, the absence of the ataxic component and the predominant presence of sensory alterations of fine fiber, nerve thickening and important epidemiological factor for leprosy,led to the suspicion and investigation of this disease by nerve biopsy that was suggestive of leprosy. After three months, in a new patient examination "to perform a biopsy in areas of anesthesia" to reinforce the diagnosis, there was the appearance of extensive slightly hypochromic lesions in the trunk and lower limbs, whose biopsy defined the diagnosis of leprosy.(AU)

Peripheral Nerve Stimulation for Painful Mononeuropathy Secondary to Leprosy: A 12-Month Follow-Up Study.

OBJECTIVE: Leprosy affects approximately 10-15 million patients worldwide and remains a relevant public health issue. Chronic pain secondary to leprosy is a primary cause of morbidity, and its treatment remains a challenge. We evaluated the feasibility and safety of peripheral nerve stimulation (PNS) for painful mononeuropathy secondary to leprosy that is refractory to pharmacological therapy and surgical intervention (decompression). METHODS: Between 2011 and 2013 twenty-three patients with painful mononeuropathy secondary to leprosy were recruited to this prospective case series. All patients were considered to be refractory to optimized conservative treatment and neurosurgical decompression. Pain was evaluated over the course of the study using the neuropathic pain scale and the visual analog scale for pain. In the first stage, patients were implanted with a temporary electrode that was connected to an external stimulator, and were treated with PNS for seven days. Patients with 50% or greater pain relief received a definitive implantation in the second stage. Follow-ups in the second stage were conducted at 1, 3, 6, and 12 months. RESULTS: After seven days of trial in the first stage, 10 patients showed a pain reduction of 50% or greater. At 12-month follow-up in the second stage, 6 of the 10 patients who underwent permanent device implantation showed a pain reduction of 50% or greater (75% reduction on average), and two patients showed a 30% reduction in pain. Two patients presented with electrode migration that required repositioning during the 12-month follow-up period. CONCLUSIONS: Our data suggest that PNS might have significant long-term utility for the treatment of painful mononeuropathy secondary to leprosy. Future studies should be performed in order to corroborate our findings in a larger population and encourage the clinical implementation of this technique.

Evaluation of polymerase chain reaction in nerve biopsy specimens of patients with Hansen's disease.

BACKGROUND: Pure neuritic variety of leprosy (PNL) presents as peripheral neuropathy with absent skin lesions and negative skin smears. Diagnosing PNL is an uphill task as most of these patients have nonspecific changes on nerve biopsy. In such circumstances, additional molecular diagnostic tools like polymerase chain reaction (PCR) has proven to be useful in diagnosing leprosy. The present study was planned to evaluate the role of PCR in nerve biopsy specimens of patients with PNL. METHODS: Patients attending the neuromuscular clinic from January 2013 to June 2014 with mononeuropathy multiplex underwent detailed diagnostic evaluation to ascertain the cause of neuropathy. Patients where this evaluation failed to establish an etiology underwent a nerve biopsy. RESULTS: Nerve biopsy was done in 52 patients, of which 35 were diagnosed as pure neuritic leprosy. Definite leprosy with positive wade fite staining for lepra bacilli was seen in 13 patients and 22 biopsies revealed a probable leprosy without lepra bacilli being identified. PCR for M. leprae was positive in 22 patients (62%). 12 of the 13 cases with definite leprosy on histopathology were PCR positive while in the AFB negative group, PCR was positive in 10 cases. PCR had a sensitivity of 92.3%, specificity of 54.5%. The positive and negative predictive value of PCR was 54.5% and 92.3% respectively. CONCLUSIONS: PCR helps in diagnosing PNL in doubtful cases. A positive PCR increases the sensitivity of detection of M. leprae especially in cases of probable PNL group where AFB cannot be demonstrated on histopathology.

Nerve biopsy in Indian patients with mononeuropathy multiplex of undetermined etiology.

INTRODUCTION: A diagnosis of mononeuropathy multiplex (MM) requires detailed evaluation to determine etiology. We performed nerve biopsy on patients with MM in whom the etiology could not be established via other investigations. METHODS: Sixty-eight patients with MM seen between January 2013 and June 2014 underwent detailed diagnostic evaluation. Those in whom the investigations failed to establish an etiology underwent nerve biopsy. RESULTS: A diagnosis of leprosy was confirmed in 14 patients and was highly probable in 17 others. Eleven patients had vasculitic neuropathy, and in 1 patient there were amyloid deposits on nerve biopsy. CONCLUSIONS: In 43 of 68 Indian patients (63%) with MM, nerve biopsy identified a definite (26 patients) or probable (17 patients) etiology. Nerve biopsy is a valuable investigation in MM that frequently results in a diagnosis of leprosy in India. Muscle Nerve, 2016 Muscle Nerve 55: 23-27, 2017.

Neuropathy in a cohort of restless leg syndrome patients.

This study aims to evaluate the types of neuropathy in a cohort of restless leg syndrome (RLS) patients and compare them with primary RLS. RLS symptoms can occur in peripheral neuropathy and may cause diagnostic confusion, and there is a paucity of studies comparing neuropathic RLS and primary RLS. Patients with RLS diagnosed according to the international restless legs syndrome study group criteria were categorized as primary RLS or secondary. Those with evidence of peripheral neuropathy were categorized as neuropathic RLS. The demographic, clinical, laboratory profile and therapeutic response to dopamine agonists at 6 months and 1 year of neuropathic RLS patients were compared between primary and secondary RLS patients. There were 82 patients with RLS of whom 22 had peripheral neuropathy and 28 had primary RLS. The etiology of neuropathic RLS was diabetes mellitus in 13, renal failure in six, hypothyroidism in five, demyelinating in two, nutritional deficiency in three, leprosy in one, and miscellaneous etiologies in four patients. The neuropathic RLS patients were older (46.0±14.1 versus 35.8±15.4 years), had shorter duration of illness (1.4±1.4 versus 6.2±6.2 years) and were more frequently symptomatic. RLS symptoms were asymmetric in primary RLS patients compared to neuropathic RLS (25% versus 0%). The therapeutic response was similar in both groups.

Leprosy neuropathy evaluated by NCS is independent of the patient's infectious state.

INTRODUCTION: Leprosy causes nerve injury, which mimics clinical and neurophysiological conditions, rendering it an excellent model of peripheral neuropathy. METHODS: A retrospective study including 822 nerve conduction studies (NCS) of 509 patients was developed to appraise the electrophysiological pattern of leprosy neuropathy. NCS of motor and sensory nerves performed before, during, and after multidrug therapy (MDT) were analyzed. RESULTS: During the three periods of MDT, while NCS alterations were similar regarding extension, topography, damage severity, and type of lesion, NCS showed that sensory was more frequent (sural nerve) (92-96%) than motor impairment (70-77%) (ulnar nerve). CONCLUSION: Once axonal loss has been installed, nerve function is little affected by inflammatory, immune and/or bacterial events since chronic neuropathy has been established, inevitably leading to the well-known leprosy sequelae occurring at any time before and/or after leprosy diagnosis.

Estudos de condução nervosa no nervo tibial através do tunel do tarso em pacientes de hanseníase / Nerve conduction studies of the tibial nerveacross the tarsal tunnel in leprosy patients

Os autores avaliaram todos os exames de condução nervosa do nervo tibial dos pacientes com suspeita de neuropatia da hanseníase, aguda ou subaguda, atendidos no Ambulatório de Hansenologia do Instituto Lauro de Souza Lima (ILSL) no período de dois anos. Foram incluídos 75 pacientes, 52 masculinos e 23 femininos, com média de idade de 44,5 anos (21 a 73 anos), totalizando 150 nervos. Procurou-se caracterizar o comprometimento neurofisiológico individualizando-se os ramos plantar medial (PM) e plantar lateral (PL), observou-se que o mais envolvido foio PL com 57,4%, seguido do PM com 42,6%. O tipo de lesão nervosa mais frequente foi a de predomínio axonal, com 66%, seguida pela mielínica, com 28,7%.O envolvimento mais freqüente e desproporcional dor amo PL, além de evidenciar o caráter compressivo do comprometimento do tibial no túnel do tarso, remete a uma mononeuropatia múltipla compressiva nos membros inferiores. A alta prevalência do comprometimento do nervo tibial foi considerada uma marcada doença, da mesma forma que a neuropatia ulnar.

The authors assessed all tibial nerve conduction studies (NCS) of the patients under suspicious of acute or subacute leprosy neuropathy, who have been attended the Leprosy Ambulatory Clinic of the ILSL during a period of two years. Seventy-five patients have been included as follows: 52 male and 23 female, between 21 and 73 years old, with the mean age of 44.5 totaling 150 nerves The medial plantar (MP) and lateral plantar ( (LP) branches were studied separately. The most involved was the LP with 57.4%, followed bythe MP with 42.6%. The most frequent injury among the abnormal nerves was the axonal lesion with 66%, followed by the myelin lesion with 28.7%. The most frequent and disproportional involvement of thePL branch not only demonstrates the compressivecharacter of the tibial nerve injury in the tarsaltunnel but also indicates a multiple entrapment mononeuropathy in the lower limbs. The high prevalence of the tibial nerve injury was considered a hallmark of the disease, as well as the ulnar neuropathy.

Mononeuropatía múltiple por lepra: descripción de un caso con presentación atípica / Mononeuritis multiplex due to leprosy: A case of atypical presentation

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Mononeuritis multiplex and painful ulcers as the initial manifestation of hepatitis B infection.

Hepatitis B virus infection leads to multisystem manifestations owing to involvement of kidney, skin, vasculature, haematopoietic and nervous system. The hepatitis B infection can cause neuropathy either to vasculitis associated with polyarteritis nodosa or immune-mediated neural damage. In this submission, we report a young woman, who presented with mononeuritis multiplex and painful ulcerations as the first manifestation of chronic hepatitis B virus infection. The antiviral therapy along with steroids led to remarkable recovery. The clinical settings of hepatitis B virus infection should not be ignored in the presentation of mononeuritis multiplex with ulcers, although the commonest cause is leprosy in the Indian sub-continent.

An unusual cause of mononeuritis multiplex.

A middle-aged man of South Asian decent presented with a 4-month history of bilateral sensory disturbance affecting the median nerve distribution and dorsum of both feet. Neurological examination was otherwise normal. A patchy absence of sensory responses was noted on nerve conduction studies and electromyogram (NCS/EMG). Over the next 3 months sensory symptoms progressed to involve median, radial, ulnar, sural and peroneal nerves bilaterally. Repeat NCS/EMG confirmed a mononeuritis multiplex predominantly involving the sensory fascicles. Areas of hypopigmentation, a right-lower motor facial weakness and ophthalmic branch trigeminal nerve involvement were noted on examination. Punch skin biopsy as well as sural nerve biopsy demonstrated chronic granulomatous inflammation without evidence of Mycobacterium. A slit skin smear test demonstrated Mycobacterium leprae consistent with a diagnosis of primary neuritic leprosy. In the appropriate clinical context, leprosy should be included in the differential diagnosis of mononeuritis multiplex.

Plantar pressure and daily cumulative stress in persons affected by leprosy with current, previous and no previous foot ulceration.

Not only plantar pressure but also weight-bearing activity affects accumulated mechanical stress to the foot and may be related to foot ulceration. To date, activity has not been accounted for in leprosy. The purpose was to compare barefoot pressure, in-shoe pressure and daily cumulative stress between persons affected by leprosy with and without previous or current foot ulceration. Nine persons with current plantar ulceration were compared to 15 with previous and 15 without previous ulceration. Barefoot peak pressure (EMED-X), in-shoe peak pressure (Pedar-X) and daily cumulative stress (in-shoe forefoot pressure time integral×mean daily strides (Stepwatch™ Activity Monitor)) were measured. Barefoot peak pressure was increased in persons with current and previous compared to no previous foot ulceration (mean±SD=888±222 and 763±335 vs 465±262kPa, p<0.05). In-shoe peak pressure was only increased in persons with current compared to without previous ulceration (mean±SD=412±145 vs 269±70kPa, p<0.05). Daily cumulative stress was not different between groups, although persons with current and previous foot ulceration were less active. Although barefoot peak pressure was increased in people with current and previous plantar ulceration, it did not discriminate between these groups. While in-shoe peak pressure was increased in persons with current ulceration, they were less active, resulting in no difference in daily cumulative stress. Increased in-shoe peak pressure suggests insufficient pressure reducing footwear in persons with current ulceration, highlighting the importance of pressure reducing qualities of footwear.

Foot impairments and limitations in walking activities in people affected by leprosy.

OBJECTIVE: To explore the relationships between perceived limitations in walking-related daily activities, walking ability (capacity), and the amount of daily walking (performance) in persons affected by leprosy and to identify their determinants. DESIGN: A cross-sectional study. SUBJECTS: Thirty-nine persons affected by leprosy. METHODS: Perceived limitations were assessed with the World Health Organization Disability Schedule II, domain "getting around". Walking capacity was assessed as covered distance in 6 min. Walking performance was recorded as mean strides/day with the Stepwatch(TM) 3 Activity Monitor. Potential determinants were sensory function, foot deformities, joint mobility, ankle muscle strength and co-morbidity. RESULTS: Perceived limitations in walking-related activities were significantly correlated with walking capacity (r = -0.47; p < 0.01) but not with walking performance, although walking capacity significantly correlated with walking performance (r = 0.38; p < 0.05). Various foot impairments independently contributed to reduced walking capacity and, to a lower degree, to perceived limitations in activities and performance. CONCLUSION: People affected by leprosy perceive limitations in walking-related activities that are determined by a reduced walking ability and the severity of foot impairments. Since perceived limitations in walking-related activities were not related to walking performance, perceived limitations are apparently weighted against the individual's needs.

O paciente com suspeita de hanseníase primariamente neural / The patient with suspicion of primarily neural leprosy

The author presents the definition of primarily neural leprosy, describes it clinically and exposes the differentiation among the similar neuropathies. The main causes of mononeuropathies, multiple mononeuropathies and polineuropathies that can be misinterpreted as leprosy were discussed. The role of nerve biopsy in the diagnosis confirmation and its prevailing findings were presented. And finally, there were suggestions of the procedures for health workers when facing these patients on the field.

[The painful multiple mononeuropathy of acute onset in the left arm which was diagnosed as leprous neuropathy].

A 31-year-old man from Myanmar with leprous neuropathy was reported. The progress of the disease was subacute but the painful symptom at the time of the onset was acute. Multiple mononeuropathy was diagnosed by the biopsy findings of the left superficial radial nerve. He was admitted to our hospital with the complaint of the weakness of his left hand and fingers which were very painful and got worse in several weeks. Motor palsy was observed in his left ulnar, median, and radial nerves, and there was the hypesthesia or anesthesia in his left hand, forearm and the medial side of his left upper arm. On nerve conduction studies, the amplitudes of CMAP and SNAP severely diminished or not detected. The pattern was compatible with multiple mononeuropathy. The biopsy of the left superficial radial nerve was performed. The pathological findings were the destruction of nerve fascicles, replacement of nerve fibers with inflammatory cells, and Mycobacterium leprae was found with the specific stain. These findings confirmed the diagnosis of the leprous neuropathy. Leprous neuropathy is one of the commonest causes of infectious neuropathy in the world, especially in Southeast Asia. These days many foreign workers from that area are staying in Japan, and the chances to see the disease are increasing. We have to recognize leprous neuropathy as a candidate for the multiple mononeuropathy of acute onset with painful dysesthesia similar to vascular neuropathy.